Isolated pemphigus vegetans of the tongue

Introduction

Pemphigus vegetans is a rare clinical variant of pemphigus groups of disorders accounting for 1 to 2 percent of all cases.[1] It predominantly affects middle aged women and is characterized by hypertrophic granulation tissue and vegetative skin lesions involving the axillae, groins and flexures.[2] This variant often affects oral mucosa. Tongue involvement in form of cerebriform tongue[2] or scrotal tongue[2] has been described.

Case Report

Recently a 35-year-old Indian woman was referred to us with 7 years history of vegetative growth on her tongue. She had not taken any treatment for it. Fifteen years back she had biopsy proven oral and skin lesions of pemphigus vulgaris for which she was treated with systemic corticosteroids for 1 year with complete recovery. She had been asymptomatic in the intervening period.

Examination showed hypertrophic vegetative grey plaque on the dorsum of her tongue [Figure - 1], with a number of erythematous eroded areas in the surrounding. Her skin and rest of the oral cavity were normal.

Biopsy from tongue lesion showed marked hyperkeratosis and acanthosis, intraepidemal bullae with acantholytic cells, neutrophils and eosinophils confirming diagnosis of pemphigus vegetans. Immuno-fluorescence couldn′t be done because of lack of facility. Normal investigations included complete hemogram, serum electrolytes, blood sugar, blood urea, serum creatinine and liver function tests.

The possibility of pyostomatitis vegetans[4],[5] was ruled out in view of the histopathological findings and the absence of inflammatory bowel disease.

Patient was treated with oral prednisolone 30 mg/day with complete disappearance of tongue lesions in 3 months.

Discussion

This case is presented because of its rarity and the fact pemphigus vegetans persisted on tongue for seven years without recurrence of lesions on any part of the body.